Publications (201)518.54 Total impact

[Show abstract][Hide abstract]ABSTRACT:
Melorheostosis is a progressive hyperostotic bone disease that commonly affects the appendicular skeleton. Melorheostosis has a significant degree of overlap with other hyperostosis conditions including osteopoikilosis and likely represent varying degrees of a clinical spectrum.
This is a report of 2 patients with melorheostosis who presented with different clinical presentations and involvement of different anatomic locations.
One of the patients presented with foot size asymmetry along with intermittent foot pain and limping. This patient also had irregular nonblanching yellow plaque-like lesion on the forehead. The second patient presented with abnormal gait but no pain. Radiographs demonstrated endosteal hyperostosis, which were consistent with a mixed picture of melorheostosis and osteopoikilois. Genetic testing of the LEMD3 gene from a blood sample was negative in both cases.
The diagnosis of melorheostosis can be made based on the clinical and radiographic features and can be challenging to differentiate from other hyperostosis conditions.
Level V.

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Aicardi syndrome (ACS) is a rare neurodevelopmental disorder that was classically characterized by the triad of agenesis of corpus callosum, infantile spasms, and chorioretinal lacunae. As new cases emerge and new common phenotypic features are being described in subsequent reports, new modified diagnostic criteria have been proposed that now classify the observed costovertebral abnormalities as supporting diagnostic features. To our knowledge there are no previous studies focusing and describing the scoliosis observed in children with ACS.
We screened billing lists to identify patients seen in the Division of Orthopaedic Surgery at our institution with a diagnosis of ACS that were treated for scoliosis after 2001. A total of 5 patients were identified. Medical records and radiographs were retrospectively reviewed in all cases. In all of the patients the diagnosis of ACS had been confirmed through complete genetic evaluation and advanced imaging.
The mean age when scoliosis was first noticed was 3.9±4.2 years (range, 0.5 to 10.5 y) with a mean Cobb angle of 22.5±6.7 degrees (range, 10 to 27 degrees). The mean age at the first orthopedic visit was 5.8±5.0 years (range, 1.5 to 13 y) with a progressed mean Cobb angle of 39.5±17.3 degrees (range, 15 to 57 degrees). Congenital vertebral anomalies were observed in 1 patient. Three patients were treated surgically; 1 of the 3 patients had a surgical complication with loss of intraoperative neuromonitoring signals. Two patients had not undergone surgery at the last visit with a mean Cobb angle of 75.5 degrees. The mean postoperative follow-up for the surgical group (cases 1 to 3) was 3±3.6 years (range, 0.6 to 7.2 y) and the mean total follow-up for both groups was 6.6±2.5 years (range, 2.6 to 8.6 y).
Scoliosis in ACS can represent a clinically significant problem that is underdiagnosed and overshadowed by the other severe medical complications associated with the syndrome. Our data suggest that scoliosis in patients with ACS is rapidly progressive and bracing is ineffective; early screening, close observation, and low threshold for referral to an orthopedic surgeon are crucial.
Level IV-case series.

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Synovial chondromatosis is a benign disorder characterized by the formation of multiple cartilaginous nodules in the synovium of large joints. Synovial chondromatosis of the spine is very rare; this is the first reported case in the skeletally immature pediatric spine. A 12-year-old female presented with a posteriolateral neck mass and discomfort. A two-stage surgical resection of the mass was performed. This case shows that synovial chondromatosis can occur in the immature pediatric spine. Synovial chondromatosis should be included in the differential diagnosis of a calcified paravertebral or lateral neck mass in children.

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The management of proximal femoral deformity in fibrous dysplasia (FD) is a challenge to the orthopaedic surgeon. The purpose of this study was to analyze the various presentations of FD of proximal femur and the results of the various treatment modalities for the same.
This is a retrospective cohort study of 23 patients (24 femora) with FD who underwent surgery for the proximal femur. The study sample included 14 males, nine females. Ten patients had a monostotic disease, eight patients had polyostotic disease, and five patients had McCune-Albright syndrome.
Group 1: shepherd crook deformity-included five patients who underwent femoral neck osteotomy. Four patients had intramedullary (IM) nailing with neck cross-pinning and all patients showed union. One patient was stabilized with external fixation, which failed. Group 2: nine patients (ten femora) presented with frank pathological fracture. Nine underwent fixation with IM nailing, one with locking plate and screws. Three patients had to undergo more than one procedure and all fractures showed good union. Group 3: nine patients who presented with bone cyst and pain. All patients underwent biopsy; four of them had curettage with bone graft.
Shepherd crook deformity can be treated by a well-planned osteotomy and fixation with intramedullary implants with neck cross-pinning. Frank pathological fractures fixation with an intramedullary nail has excellent results even if not accompanied by resolution of the fibrodysplastic lesion. More than one procedure may be required. External fixation is not an optimal choice for fixation of femoral osteotomies in FD.

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Chest wall osteochondroma is a rare tumor in children. Even though the potential for malignant transformation or serious intrathoracic complications is low, it has led some centers to advocate surgical management of these bony tumors. We present our experience of the surgical management of costal osteochondromata.

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Study Design
Consensus-based creation of a checklist and guideline.
Objective
To develop a consensus-based checklist to guide surgeon responses to intraoperative neuromonitoring (IONM) changes in patients with a stable spine and to develop a consensus-based best practice guideline for IONM practice in the United States.
Summary of Background Data
Studies show that checklists enhance surgical team responses to crisis situations and improve patient outcomes. Currently, no widely accepted guidelines exist for the response to IONM changes in spine deformity surgery.
Methods
After a literature review of risk factors and recommendations for responding to IONM changes, 4 surveys were administered to 21 experienced spine surgeons and 1 neurologist experienced in IONM. Areas of equipoise were identified and the nominal group process was used to determine items to be included in the checklist. The authors reevaluated and modified the checklist at 3 face-to-face meetings over 12 months, including a period of clinical validation using a modified Delphi process. The group was also surveyed on current IONM practices at their institutions. This information and existing IONM position statements were used to create the IONM best practice guideline.
Results
Consensus was reached for the creation of 5 checklist headings containing 26 items to consider in the response to IONM changes. Consensus was reached on 5 statements for inclusion in the best practice guideline; the final guideline promotes a team approach and makes recommendations aimed at decreasing variability in neuromonitoring practices.
Conclusions
The final products represent the consensus of a group of expert spine surgeons. The checklist includes the most important and high-yield items to consider when responding to IONM changes in patients with a stable spine, whereas the IONM guideline represents the group consensus on items that should be considered best practice among IONM teams with the appropriate resources.

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Postural orthostatic tachycardia syndrome (POTS) is the most common of several types of dysautonomia, characterized by dysfunction of the autonomic nervous system manifesting with symptoms of orthostatic intolerance with or without associated orthostatic hypotension and excessive autonomic excitation. Given the numerous presenting musculoskeletal symptoms of POTS and its known associations with other clinical entities like Ehlers-Danlos syndrome, POTS constitutes an unusual treatment challenge of which the orthopaedic surgeon and other related healthcare providers should be aware.

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The use of the conventional halo is accompanied by the possibility of serious complications, especially in the pediatric population. Complications could include penetration of pin into the skull, pin loosening, pin tract infection, cranial nerve palsies, and vest-related pressure sores. The noninvasive "pinless" halo was introduced in an attempt to mitigate these problems while retaining the effectiveness of the conventional halo. The purpose of this study is to determine the indications and complications related to pinless halo application.

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Unicameral bone cysts are benign bone lesions that are often asymptomatic and commonly develop in the proximal humerus and femur of skeletally immature patients. The etiology of these lesions remains unknown. Most patients present with a pathologic fracture, but these cysts can be discovered incidentally, as well. Radiographically, a unicameral bone cyst appears as a radiolucent lesion with cortical thinning and is centrally located within the metaphysis. Although diagnosis is frequently straightforward, management remains controversial. Because the results of various management methods are heterogeneous, no single method has emerged as the standard of care. New minimally invasive techniques involve cyst decompression with bone grafting and instrumentation. These techniques have yielded promising results, with low rates of complications and recurrence reported; however, prospective clinical trials are needed to compare these techniques with current evidence-based treatments.

The Journal of the American Academy of Orthopaedic Surgeons 05/2014; 22(5):295-303. DOI:10.5435/JAAOS-22-05-295 · 2.40 Impact Factor

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Adolescent idiopathic scoliosis (AIS) is a common rotational deformity of the spine that presents in children worldwide, yet its etiology is poorly understood. Recent genome-wide association studies (GWAS) have identified a few candidate risk loci. One locus near the chromosome 10q24.31 LBX1 gene (OMIM #604255) was originally identified by a GWAS of Japanese subjects and replicated in additional Asian populations. To extend this result, and to create larger AIS cohorts for the purpose of large-scale meta-analyses in multiple ethnicities, we formed a collaborative group called the International Consortium for Scoliosis Genetics (ICSG).
Here, we report the first ICSG study, a meta-analysis of the LBX1 locus in six Asian and three non-Asian cohorts.
We find significant evidence for association of this locus with AIS susceptibility in all nine cohorts. Results for seven cohorts containing both genders yielded P=1.22×10-43 for rs11190870, and P=2.94×10-48 for females in all nine cohorts. Comparing the regional haplotype structures for three populations, we refined the boundaries of association to a ∼25 kb block encompassing the LBX1 gene. The LBX1 protein, a homeobox transcription factor that is orthologous to the Drosophila ladybird late gene, is involved in proper migration of muscle precursor cells, specification of cardiac neural crest cells, and neuronal determination in developing neural tubes.
Our results firmly establish the LBX1 region as the first major susceptibility locus for AIS in Asian and non-Hispanic white groups, and provide a platform for larger studies in additional ancestral groups.

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Cervical spondylolysis with spondylolisthesis is a rare condition in the pediatric population. The nature of this condition and clinical presentation are important to provide appropriate management.
This is a case report of 3 adolescent siblings who had C6 cervical spondylolysis with spondylolisthesis.
Two cases were diagnosed insidiously with absence of neurological deficits and no instability on imaging and were treated conservatively. One case had a traumatic presentation associated with instability on imaging and was managed with cervical fusion and instrumentation. All 3 patients were doing well on a follow-up of >2 years.
These cases suggest that cervical spondylolysis could be familial. The treatment was offered based on clinical presentation and presence of instability on radiographic studies.
Level IV.

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Retrospective cohort study using the Pediatric Health Information Systems (PHIS) database.
To determine the association between antifibrinolytic use and red cell transfusions in spinal fusion operations performed at 37 US Children's Hospitals.
Evidence from randomized clinical trials and systematic reviews suggests that antifibrinolytic therapy can significantly reduce blood loss in children undergoing scoliosis surgery, but the effectiveness of these agents as used in surgeries performed at US children's has not been studied.
We included children 0-18 years of age with diagnoses indicating adolescent idiopathic scoliosis (AIS) or neuromuscular scoliosis (NMS) for whom a spinal fusion procedure was performed between 1/1/2006-9/30/2009. Patients with malignancy, trauma, coagulation disorders, or for whom a cell salvage device was employed were excluded. Multilevel logistic regression was used to determine associations between ε-aminocaproic acid (EACA), tranexamic acid (TXA) and aprotinin (APR) use and blood transfusions, controlling for patient and surgery characteristics.
Cohorts consisted of 2722 AIS and 1547 NMS procedures. Antifibrinolytic use varied across hospitals (AIS 3.3%, IQR 0%-42%; NMS 12 IQR 0%-46%), and was significantly associated with NMS, posterior fusion, number of vertebrae fused. Overall, 15% of children received EACA, 7% TXA, and 2% APR. The median hospital-specific rate of red cell transfusions was 24% for AIS, and 43% for NMS. In AIS operations, EACA use, but not TXA use, was associated with significantly lower odds of transfusion (OR 0.42, P<0.001 vs. OR 1.0, P=0.8). In NMS operations, neither EACA nor TXA use was associated with a decrease in odds of red cell transfusions.
The effectiveness of antifibrinolytics as used outside of clinical trials is unclear and should continue to be explored. Future prospective research is needed to evaluate which administration protocols will most benefit patients, as well as to determine the comparative effectiveness of these drugs in the context of other blood conservation strategies.

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Interest in developing national health care has been increasing in many fields of medicine, including orthopaedics. One manifestation of this interest has been the development of global health opportunities during residency training.
We assessed global health activities and opportunities in orthopaedic residency in terms of resident involvement, program characteristics, sources of funding and support, partner site relationships and geography, and program director opinions on global health participation and the associated barriers.
An anonymous 24-question survey was circulated to all US orthopaedic surgery residency program directors (n = 153) by email. Five reminder emails were distributed over the next 7 weeks. A total of 59% (n = 90) program directors responded.
Sixty-one percent of responding orthopaedic residencies facilitated clinical experiences in developing countries. Program characteristics varied, but most used clinical rotation or elective time for travel (76%), which most frequently occurred during Postgraduate Year 4 (57%) and was used to provide pediatric (66%) or trauma (60%) care. The majority of programs (59%) provided at least some funding to traveling residents and sent accompanying attendings on all ventures (56%). Travel was most commonly within North America (85%), and 51% of participating programs have established international partner sites although only 11% have hosted surgeons from those partnerships. Sixty-nine percent of residency directors believed global health experiences during residency shape future volunteer efforts, 39% believed such opportunities help attract residents to a training program, and the major perceived challenges were funding (73%), faculty time (53%), and logistical planning (43%).
Global health interest and activity are common among orthopaedic residency programs. There is diversity in the characteristics and geographical locations of such activity, although some consensus does exist among program directors around funding and faculty time as the largest challenges.

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Study Design: Therapeutic Level II Cohort Study.Objective: To evaluate the safety of adjunctive local application of vancomycin powder (VP) for infection prophylaxis in posterior instrumented thoracic and lumbar spine wounds in pediatric patients >25kg.Summary of Background Data: Spine surgeons have largely turned to vancomycin prophylaxis in an attempt to decrease the incidence of late surgical site infection (SSI) and acute SSI from methicillin resistant Staphylococcus aureus (MRSA). In adult patients the adjunctive local application of VP with an IV cephalosporin has been shown to significantly decrease postsurgical wound infection rates; however, the safety of VP as an adjunct in pediatric spine surgery has not been reported.Methods: We reviewed data collected under a systematic protocol specifically designed to monitor the safety profile of VP. We measured changes in creatinine and systemic vancomycin levels following intrawound application of 500mg of unreconstituted VP during spine deformity correction surgery in patients >25kg (patients also received routine IV cephalosporin prophylaxis). Lab values were measured preoperatively and on postoperative days (POD) 1 and 4. Any adverse reactions and infections through available follow-up (2-8 months) were recorded.Results: 87 consecutive pediatric spinal deformity patients >25kg who received intraoperative VP over a 9 month period were identified. 63% of the patients in this series had adolescent idiopathic scoliosis, 15% congenital scoliosis, 15% neuromuscular scoliosis, and 5% spondylolisthesis. The average change in creatinine levels between the preoperative and POD 1 draw was -0.03 and between the preoperative and POD 4 draw was -0.075. The postoperative systemic vancomycin levels remained undetectable. None of the patients experienced nephrotoxicity or red man syndrome. 3/87 patients developed a SSI.Conclusion: In this cohort there were no clinically significant changes in creatinine level or systemic vancomycin level caused by use of intraoperative VP.

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Vascular malformations are abnormal proliferations of the endothelial lining of the blood vessels that are most commonly found in skin and can penetrate the local soft tissue and muscle. Only 1% of all vascular malformations are found in the bone. Skeletal vascular malformations of the long bones, including the humerus, are extremely rare entities that are particularly uncommon in children. Only 4 reports involving the humerus have been described in children previously. We present the case of an intraosseous capillary vascular malformation of the right distal humerus in a 3-year-old boy. Definitive treatment was successful with a single operation using curettage and intramedullary decompression with grafting of an osteoconductive calcium sulfate pellet filler and autogenous bone graft. This is, to our knowledge, the youngest reported case of a solitary intraosseous capillary vascular malformation involving the distal humerus.

[Show abstract][Hide abstract]ABSTRACT:
Chondroblastoma and chondromyxoid fibroma are benign but locally aggressive bone tumors. Chondroblastoma, a destructive lesion with a thin radiodense border, is usually seen in the epiphysis of long bones. Chondromyxoid fibroma presents as a bigger, lucent, loculated lesion with a sharp sclerotic margin in the metaphysis of long bones. Although uncommon, these tumors can be challenging to manage. They share similarities in pathology that could be related to their histogenic similarity. Very rarely, chondroblastoma may lead to lung metastases; however, the mechanism is not well understood.

The Journal of the American Academy of Orthopaedic Surgeons 04/2013; 21(4):225-33. DOI:10.5435/JAAOS-21-04-225 · 2.40 Impact Factor